AB1281 PRESENTATION, TREATMENT AND PROGNOSIS OF SARCOIDOSIS IN A COHORT OF SPANISH PATIENTS
Authors: A. I. Rebollo Giménez, L. Sacristán-Bou, D. Bellido Pastrana, P. Mata Calderón, I. Domínguez Osorio, I. M. Ordóñez Dios, M. Orta Caamaño, C. Bujalance Cabrera, F. J. Lázaro Polo
Journal: Annals of the Rheumatic Diseases (2022)

Background Sarcoidosis is usually a benign disease, but it is described that the lung is the most frequently affected organ and in some cases the disease can progress to pulmonary fibrosis. Systemic corticosteroids, immunosuppressants and/or monoclonal antibodies are used for its treatment. The mortality rate without treatment is 1% to 6%. Objectives To establish the frequency of appearance of the stages of radiological involvement in patients diagnosed with sarcoidosis at Ciudad Real University General Hospital (CRUGH) for 4 years, describe the treatment guidelines used and investigate the causes of death. Methods Retrospective descriptive study that includes patients treated at Pneumology, Rheumatology and Internal Medicine Services of CRUGH from January 2017 to January 2021. Sarcoidosis diagnosis has been established according to ATS/ERS/WASOG (1999) criteria: compatible clinical and radiological presentation, evidence of non-caseating granulomas and exclusion of other granulomatous disease. The variables have been collected in an anonymized database analyzed by SPSS program. Results We have included 47 patients, 24 men and 23 women, with 52.0 ± 14.3 years of age. All presented radiological pulmonary involvement and in 26 cases (55.3%) there were also extrapulmonary manifestations. The most frequent radiological pattern is stage II in 29 cases (67.7%, hilar adenopathies and pulmonary infiltrates); followed by stage I in 16 (34.1%, hilar adenopathies) and only 2 cases presented stage IV (4.2%, fibrosis). Currently, 20 patients (42.5%) are without treatment: 9 (19.1%) have not required it due to hyliomediastinal lymph node involvement without associated symptoms, and 11 (23.4%) achieve remission with oral glucocorticoids [OCG] at doses recommended in clinical guidelines. The other 27 (57.5%) receive these regimens: 10 (21.3%) OCG at usual doses; 11 (23.4%) OCG and conventional immunosuppressants -metrotexate 9 (19.1%), azathioprine 1 (2.1%) and cyclophosphamide 1 (2.1%)-; 3 (6.4%) OCG and antimalarials (hydroxychloroquine); 2 (4.2%) monotherapy with azathioprine; and 1 (2.1%) biological treatment with infliximab. Three patients died (6.4%): one aged 71 due to progression to pulmonary fibrosis (2.1%), another 2 due to pulmonary adenocarcinoma (47 years old) and due to adenocarcinoma of the rectum (81 years old). Conclusion The predominant radiological pattern in our cohort is stage II with hilar adenopathies and pulmonary infiltrates. The most used treatment is OCG, as monotherapy (44.7%) or combined (29.4%). The high frequency of cancer mortality stands out. References [1]Drent M, Crouser ED, Grunewald J. Challenges of Sarcoidosis and Its Management. N Engl J Med. 2021 Sep 9;385(11):1018-1032 [2]Crouser ED, Maier LA, Wilson KC et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 Apr 15;201(8):e26-e51. [3]Rahaghi FF, Baughman RP et al. Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur Respir Rev. 2020 Mar 20;29(155):190146. [4]Hunninghake GW, Costabel U, Ando M et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis. 1999 Sep;16(2):149-73. Disclosure of Interests Ana Isabel Rebollo Giménez: None declared, Lirios Sacristán-Bou Speakers bureau: LS has received honoraria for speaker bureaus from: Glaxosmithkline (GSK), Chiesi, Gebro Pharma, Booehringer Ingelheim, Roche, Rovi, Teva, David Bellido Pastrana Speakers bureau: DB has received honoraria for speaker bureau from Glaxosmithkline, Patricia Mata Calderón Speakers bureau: PM has received honoraria for speaker bureaus from: Novartis, GSK, Gebro, Boerhinger, Astrazeneca, Menarini, Ferrer, Isabel Domínguez Osorio: None declared, Isabel María Ordóñez Dios Speakers bureau: IO has received honoraria for speakers bureau from Novartis, GSK, boehringer ingelheim, Roche and Chiesi, Marta Orta Caamaño Speakers bureau: MO has received honoraria for speaker bureau from Glaxosmithkline and Chiesi , Carlos Bujalance Cabrera Speakers bureau: CB has received honoraria for speaker bureau from Faes and GSK, Francisco Javier Lázaro Polo Speakers bureau: FL has received honoraria for speaker bureau from GSK, Astrazeneca and Sanofi